Metastasis to hypopharynx from epidermotropic metastatic malignant melanoma.

Previous reports proposed the concept and criteria of epidermotropic metastatic malignant melanoma (EMMM): (a) dermal involvement equal to or broader than the epidermal involvement, (b) atypical melanocytes within the dermis, (c) thinning of the epidermis, (d) widening of the papillary dermis with an epithelial collarette, and (e) vascular invasion of atypical melanocytes. However, it remains unclear whether EMMM also involves the mucosal epithelium. In this case, the patient was diagnosed with EMMM based on the histopathological findings of the patient's multiple skin lesions and clinical course. The patient also developed metastasis to the hypopharynx. Although histopathological findings of the lesion suggested the possibility of melanoma in situ, as the lesion included atypical melanocytes in the mucosal epithelium, the clinical course supported the diagnosis of hypopharyngeal metastasis from EMMM. This case suggests that EMMM may have epitheliotropic features not only in the skin but also in the mucosa.

Epstein-Barr Virus Positive Diffuse Large B-Cell Lymphoma with Epidermotropism.

Primary cutaneous B-cell lymphomas are rare diseases comprising nodular to diffuse lymphoid cell infiltration with an overlying grenz zone and no epidermal involvement upon histological analysis. Diagnostics can become challenging when lymphomas exhibit the characteristics of both B and T-cells. Differential diagnoses may include reactive proliferations, cutaneous composite lymphomas, and transformed mycosis fungoides. Immunohistochemistry and gene arrangement tests may be beneficial to clarify the diagnosis. Herein, we report a rare case of epidermotropic EBV-positive cutaneous B-cell lymphoma along with a literature review.

Probable Primary Cutaneous CD8+ Aggressive Epidermotropic Cytotoxic T-cell Lymphoma: A Case Report of a Diagnostic Challenge.

Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma is a rare variety of cutaneous lymphoma. This subtype has an aggressive and quickly progressive clinical course with a survival time of 32 months from the commencement of skin lesions. This article describes a probable case of primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma in a 63-year-old female, which manifested as diffuse non-pruritic erythematous plaques and nodules. The diagnosis of this possible entity was aided by the histopathological and immunohistochemical findings, while immunohistochemistry for T-cell receptor (TCR) gamma/delta could not be done.

Pigmented purpura dermatosis-like mycosis fungoides: four case reports and a review of published cases.

Mycosis fungoides (MF) is the most prevalent type of cutaneous T-cell lymphoma and is generally characterized by multiple patches or plaques with fine scales. One of its variants manifests with multiple purpuric eruptions, mimicking benign pigmented purpuric dermatosis (PPD). To investigate clinicopathological features of PPD-like MF patients. We report four PPD-like MF cases and summarize the clinicopathological features described in reports of nine PPD-like MF cases published in the past 20 years. Compared with benign PPD, petechial lesions in PPD-like MF are more generalized, persistent, and resistant to conventional steroid treatment. Histologically, a superficial dermal band-like infiltrate of atypical lymphocytes with epidermotropism seems to be the most common feature of PPD-like MF. A lymphoid phenotype of CD4+ CD7- T cells and a monoclonal T-cell profile, demonstrated by T-cell receptor gene arrangement analysis, favour a diagnosis of PPD-like MF. Although the exact relationship between PPD and PPD-like MF remains unclear, our study has attached importance to the differential diagnosis of the two diseases in cases of overlooked MF variants. If persistent or generalized purpuric lesions are present, PPD-like MF should be taken into consideration. A thorough physical examination combined with pathological findings may lead to a correct diagnosis.

Clinicopathological Spectrum of Cutaneous Lymphomas and Distinction of Early Mycosis Fungoides from its Mimics-A Retro-Prospective Descriptive Study from Southern India.

Background: Cutaneous lymphomas (CLs) could be either primary (PCL) or secondary; the former comprises cutaneous T-cell lymphomas (CTCLs) and cutaneous B-cell lymphomas (CBCLs). Mycosis fungoides (MF) is the most common PCL. Diagnosis of early MF and distinguishing it from benign inflammatory mimics is challenging. This study aims to assess the clinicopathological spectrum of CL and to characterize early MF from its mimics using clinical characteristics, histopathological features, and ancillary techniques. Materials and Methods: This retro-prospective descriptive study was conducted in a tertiary-care institute, for over 5 years. Clinically as well as histopathologically suspected and biopsy-proven CL and their mimics were included. Cases were reviewed and subgrouped based on clinical and histopathological parameters and immunohistochemistry (IHC). Data were analyzed using descriptive statistics and a Chi-square test at a 5% level of significance. Results: Among PCL, CTCL comprised 84% (21/25) and CBCL was 16% (4/25); the most common CTCL was MF at 81% (17/21). Histologically, atypia of dermal infiltrate (100%), epidermotropism (91.7%), basal alignment of lymphocytes (91.7%), clear haloed cells (91.7%), wiry collagen (66.7%), grandiosity sign (50%), eccrine infiltration (66.7%), and follicular infiltration (50%) were significantly associated with early MF. Spongiosis (84.6%), pigment incontinence (84.6%), exocytosis (76.9%), and parakeratosis (76.9%) were significantly associated with inflammatory mimics. There was no significant difference in the downregulation pattern of CD7 (P = 0.206) between early MF and its mimics. The four cases of CBCL in our study were plasmablastic lymphoma, diffuse large B-cell lymphoma, follicular lymphoma, and lymphoblastic lymphoma. Conclusion: MF was the most common PCL. Histological parameters showed a significant difference, whereas IHC did not show any significant difference between early MF and its mimics.

Linfoma primario cutáneo de células T epidermotropo agresivo CD8+ / Primary cutaneous aggressive epidermotropic CD8 + T-cell lymphoma

El linfoma de células T epidermotropo agresivo CD8+ o linfoma de Berti es un subtipo infrecuente de los linfomas primarios cutáneos, descripto en 1999. Aún se considera una entidad provisional en la última clasificación de linfomas cutáneos primarios de la Organización Mundial de la Salud. Predomina en los hombres adultos y se manifiesta con pápulas, placas hiperqueratósicas y tumores ulcerados, diseminados y de inicio agudo. En la histopatología, es característica la presencia de un infiltrado de linfocitos atípicos medianos CD8 +, con epidermotropismo de patrón pagetoide. Tiene un comportamiento agresivo y es de mal pronóstico, con una sobrevida a los 5 años menor del 40%. Si bien esta entidad es un reto terapéutico, el tratamiento inicial se basa en poliquimioterapia y/o trasplante de células hematopoyéticas.

The aggressive epidermotropic CD8+ T-cell lymphoma or Berti's lymphoma, is a rare subtype of primary cutaneous lymphomas, first described in 1999. It is still considered a provisional entity by the latest World Health Organization classification of cutaneous lymphomas. Patients are commonly adults with a male predominance and it is characterized by widespread rapid evolving papules, hyperkeratotic annular plaques and ulcerated tumors. Histopathologicfindingsarecharacteristic,withaninfiltrate of medium-sized CD8+ atypical lymphocytes involving the epidermis with a pagetoid pattern. Ithasanaggressivebehaviorandtheprognosisispoor with a 5-yearsurvival less than 40%. Instead these disease represents a therapeutic challenge, the initial treatment consists on polychemotherapy and hematopoietic stem cell transplantation.

Utility of CD30, Ki-67, and p53 in assisting with the diagnosis of mycosis fungoides with large cell transformation.

INTRODUCTION: Mycosis fungoides (MF) with large cell transformation (LCT) is an advanced stage of cutaneous lymphoma with a poor prognosis. Identification of LCT is critical and especially challenging when the number of large abnormal lymphocytes is near but below 25%. We propose that Ki-67 and p53 may be useful in making this diagnosis. METHODS: We identified 17 patients with advanced stage (T3 or T4) MF without LCT and 38 patients with a biopsy-confirmed new diagnosis of MF with LCT treated at our institution's cutaneous lymphoma clinic from 2012 to 2016. Seventeen patients underwent 22 biopsies with advanced stage MF (control), and 38 patients with 46 biopsies of MF with LCT were included in this study. RESULTS: The MF cohort had an average CD30 expression of 4%, while the MF-LCT cohort had an average CD30 expression of 22% (P < 0.05). The MF cohort had an average Ki-67 staining of 13%, while the MF-LCT group had an average Ki-67 staining of 57% (P < 0.05). Forty-seven percent of the MF-LCT group was positive for p53; on the other hand, none of the MF control group showed increased p53 expression (P < 0.05). DISCUSSION: While CD30 shows some value in delineating large cell transformation, Ki-67 and p53 appear to be useful immunohistochemical markers in the diagnosis of LCT.

Treponema Pallidum Epidermotropism in Nodular Secondary Syphilis.

Nodular secondary syphilis results from the hematogenous and lymphatic dissemination of spirochetes. Clinically, the lesions appear as partially infiltrated plaques or red-violaceous nodules, which can be solitary or multiple. Several hypotheses have been put forward to explain the formation of these infiltrated or granulomatous lesions. Among the most accepted are the specific hypersensitivity reactions to Treponema pallidum or the lenghty duration of the disease. We present a case of nodular syphilis where immunohistochemistry revealed the presence of multiple spirochetes invading the epidermis.

Epidermotropic presentation by splenic B-cell lymphoma: The importance of clinical-pathologic correlation.

There are exceedingly rare reports of patients with epidermotropic B-cell lymphomas. A subset presented with intermittent, variably pruritic papular eruptions and involvement of their spleens, peripheral blood and bone marrow at the time of diagnosis. Furthermore, some experienced an indolent course despite dissemination of their lymphomas. We report a 66-year-old woman with a 12-year history of intermittent eruptions of non-pruritic, salmon-colored papules on her torso and proximal extremities that occurred in winter and resolved with outdoor activity in spring. Skin biopsy revealed an epidermotropic B-cell lymphoma with a non-specific B-cell phenotype and heavy chain class switching with IgG expression. On workup, our patient exhibited mild splenomegaly and low-level involvement of her peripheral blood and bone marrow by a kappa-restricted B-cell population. A splenic B-cell lymphoma was diagnosed. Considering her longstanding history and absences of cytopenias, our patient has been followed without splenectomy or systemic therapy. Furthermore, the papules have responded dramatically to narrowband UVB. Our case and a review of similar rare reports aim to raise awareness among dermatopathologists and dermatologists of a clinically distinct and indolent subset of epidermotropic splenic lymphomas with characteristic clinical and histologic findings.

A case of radiation-induced osteosarcoma of the skull presenting as a cutaneous epidermotropic tumor with a short latent period.

Radiation-induced sarcoma (RIS) is an unusual but well documented tumor. The frequency of RIS of the head and neck region has been reported as 0.143%. In the literature the median interval between irradiation and development of sarcoma is 11 years. Cases of RIS with a short latent period, that is, less than 4 years are rare. We report a case of a 34-year-old female who developed an osteosarcoma of the scalp, over a previous craniotomy scar, 3 years after excision of a frontal anaplastic oligodendroglioma which had been followed by a course of 6 weeks radiotherapy (58 Gy) and 6 cycles of temozolomide. The histological features were those of a high-grade osteosarcoma with epidermotropism of tumor cells. Lymph nodes were partially replaced by high-grade metastatic osteosarcoma, with extra-nodal lymphatic tumor thrombi. To our knowledge the only other case report of post-radiation osteosarcoma with a short latency period was a case of osteosarcoma in the craniofacial bone 3 years after radiotherapy for maxillary squamous cell carcinoma. The histological finding of prominent replacement of the epidermis by osteosarcoma has not been reported before.

Indeterminate Dendritic Cell Tumor in Thoracic Spine: A Case Report.

BACKGROUND: Indeterminate dendritic cell tumor (IDCT) is an extremely rare hematologic disorder with poorly understood pathogenesis. Occasionally encountered by hematologists, unusual presentations of IDCT have not been reported in the spine literature. METHODS: We report a 51-year-old man who presented with a 3-month history of progressively worsening axial thoracic back pain radiating to his sides. Magnetic resonance imaging revealed a 3-cm enhancing mass at the T9 vertebral body with an exophytic component causing significant canal stenosis. Initial percutaneous biopsy revealed histiocytic sarcoma. RESULTS: The patient underwent exploratory thoracotomy and en bloc resection of the lesion with T8-10 fusion. Final pathology results revealed IDCT with fibrosis. IDCT immunostaining was partially positive for Langerhans cell marker (positive for S100 and CD1a, but lacked Birbeck granules and Langerin stain) and partially positive for blastic plasmacytoid dendritic cell neoplasm. Additionally, it was positive for CD45, CD68, and CD163. Lymphadenopathy was absent in this patient. CONCLUSIONS: Although first reported in the 1980s, IDCT has been omitted from most classifications owing to its rarity. Hematologists have debated the cell of origin; it is believed to comprise pre-Langerhans cells, as Birbeck granules are acquired after migration to the epidermis. IDCT remains of indeterminate origin. We report the first case of spinal IDCT. Familiarity with the histologic features is warranted to ensure accurate diagnosis and appropriate treatment.

CD3+, CD56+, CD4-, CD8-, CD20-, CD30- Peripheral T-Cell Non-Hodgkin's Lymphoma: A Rare Case Report.

Cutaneous T-cell lymphoma (CTCL) commonly presents as mycosis fungoides or Sezary syndrome, both having CD4 positivity. A subset of CTCL which lacks CD4 surface marker is classified as cutaneous γ and δ-T-cell lymphoma (CGD-TCL). Because of its rarity and inability to study large number of patients, the impact of immunophenotype on the clinical outcome of primary CTCL in patients is limited. We report a case of primary CGD-TCL in a 71-year-old male because of this rarity and to emphasize its aggressive nature.

Primary cutaneous CD8+ cytotoxic T-cell lymphoma involving the epidermis and subcutis in a young child.

CD8+ cytotoxic T-cell lymphoma involving the skin represents a heterogeneous group of diseases that include subcutaneous panniculitis-like T-cell lymphoma, primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, and 'type D' lymphomatoid papulosis. In this report, we describe a case of CD8+ cytotoxic T-cell lymphoma involving both the epidermis and subcutis. The patient was a 6-year-old girl who presented with a 3-year history of multiple plaques on her trunk and legs. The lesions had relapsed twice but responded well to prednisone. Histopathologic examination showed the proliferation of atypical lymphocytes in the epidermis, dermis and subcutaneous tissue. On immunohistochemical analysis, the atypical lymphocytes were positive for ßF1, CD3, CD8, perforin, granzyme B and TIA-1, but negative for T-cell receptor (TCR) γ, CD4, CD30 and CD56. It was difficult to classify this tumor in terms of the known types of cutaneous lymphoma, and this case should be differentiated with subcutaneous panniculitis-like T-cell lymphoma and primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma.

Epidermotropic Metastatic Malignant Melanoma / 대한피부과학회지

Cutaneous metastases of malignant melanoma are usually confined to the dermis or subcutaneous fat. In some instances, however, they may involve the epidermis. A 68-year-old woman with a malignant melanoma on the subungual area of the right great toe presented with multiple blackish pinhead-sized macules surrounding an ulcerative lesion on the right great toe. Histopathological study of the macules showed atypical melanocytes and melanocytic nests in the papillary dermis and the dermoepidermal junction. A thinning of the epidermis, widening of the dermal papillae by aggregated atypical melanocytes, epidermal collarette formation, and angiotropism were also seen. A diagnosis of epidermotropic metastatic malignant melanoma (EMMM) was made. EMMM is a specific form of metastatic malignant melanoma that is associated with epidermotropism of melanoma cells and several histopathological features. The differential diagnosis between primary malignant melanoma and EMMM can be difficult because of their similar clinical and histological features. Here, we report a case demonstrating EMMM.

Indeterminate cell histiocytosis with naïve cells.

Histiocytoses are a heterogeneous group of disorders characterized by proliferation and accumulation of cells of mononuclear-macrophage system and dendritic cells. Histiocytoses are categorized according to the cell of origin into Langerhans cell histiocytosis (LCH), Non Langerhans cell histiocytoses and indeterminate cell histiocytosis (ICH). ICH is an extraordinary rare neoplastic dendritic cell disorder that has poorly understood histogenesis and pathogenesis. It is characterized by a proliferation of dendritic cells, which mimic Langerhans cells immunophenotypically (positive for CD1a and S-100 protein), but lack Birbeck granules characteristic of Langerhans cells. Twenty-four year-old Egyptian male was presented with reddish brown chest wall nodule. Clinical, histopathological, immunohistochemical and ultrastructure features are typical for ICH. He was in a good state without any evidence of recurrence or metastasis after 24 months follow up. Peculiar histopathological features were detected in the present case. Many unidentified cells with Hematoxylin & Eosin Langerhans like features showed negative staining for S-100, CD1a, Langerin and CD68. In absence of cellular atypia and mitosis, the infiltrating cells showed epidermotropism that was reported once in ICH as well as neural and perineural invasion that were not previously reported. Therefore we prefer using a tentatively designated diagnosis; dendritic cell tumor, not otherwise specified or newly proposed diagnosis (Indeterminate cell histocytosis with naïve cells) for the present case.

A case of lymphomatoid keratosis.

Lymphomatoid keratosis (LK) is considered to be a rare variant of cutaneous lymphoid hyperplasia, with epidermotropism. We herein report a case of LK which developed on the abdomen of an elderly Korean woman. A 60-year-old woman presented with a 10-year history of a pruritic, solitary, brown to black plaque on the abdomen. Histopathologically, the specimen showed hyperkeratosis, parakeratosis, acanthosis and Pautrier's micro-abscess in the epidermis, and a lichenoid infiltration of lymphocytes in the dermis, which expressed both B cell and T cell lineage on the immune-histochemical staining. Based on these clinical and histopathological findings, our case was diagnosed as LK. To our knowledge, this is the first case report of LK in the Korean dermatologic literature.